Bricoune Ornella, Kareem Syeda Saba, Wallace Gerald, Iacono David P, Macaulay Robert, Etame Arnold, Pina Yolanda, Robinson Timothy J, Mokhtari Sepideh
Department of Neuro-Oncology, Moffitt Cancer Center, Tampa, FL 33612, USA.
Department of Pharmacy, Moffitt Cancer Center, Tampa, FL 33612, USA.
Exp Ther Med. 2023 Jul 10;26(2):410. doi: 10.3892/etm.2023.12109. eCollection 2023 Aug.
Patients with primary central nervous system lymphoma (PCNSL) typically present with non-focal neurological symptoms, including disorientation, poor balance and memory loss with unifocal or multifocal periventricular lesions seen on MRI. Deviations from these characteristic findings can delay diagnosis and lead to additional diagnostic tests being needed. The present study reports a 68-year-old man with a recent varicella zoster infection and history of acetylcholine receptor antibody-positive myasthenia gravis who received mycophenolate mofetil for 22 years. He presented with left eye vision changes and cognitive memory deficits. A brain MRI showed an enhancing lesion within his left medulla extending to the cerebellum. Cerebrospinal fluid analysis was positive for Epstein-Barr virus (EBV) and negative for malignancy. He was diagnosed with varicella zoster virus vasculopathy. At 3 months later, a repeat brain MRI showed multiple new enhancing lesions developing bilaterally along the periventricular white matter. Soon after, he presented to a local ER with acute left-sided blurry vision and worsening memory loss, and he began receiving steroids. Because of rapid symptom progression, he underwent resection of the left frontal lesion, which showed EBV-induced diffuse large B-cell lymphoma (DLBCL). Mycophenolate mofetil was discontinued, and within 24 h of one dose of intravenous 500 mg/m rituximab, he had a dramatic improvement in left eye vision and memory loss. He experienced mixed responses to rituximab after 3 cycles. Following one dose of high-dose methotrexate, he developed subsequent chronic kidney disease and required dialysis. He received whole-brain radiation therapy with craniospinal radiation and is currently in complete remission. An EBV-induced DLBCL diagnosis should be highly considered for patients with periventricular lesions and EBV-positive cerebrospinal fluid. Misdiagnosis or delay in PCNSL diagnosis because of atypical features in disease presentation and radiographic findings could lead to PCNSL progression and worsening neurological deficits.
原发性中枢神经系统淋巴瘤（PCNSL）患者通常表现为非局灶性神经症状，包括定向障碍、平衡能力差和记忆力减退，MRI可见单灶或多灶性脑室周围病变。这些特征性表现的偏差可能会延迟诊断，并导致需要进行额外的诊断检查。本研究报告了一名68岁男性，近期患有水痘带状疱疹感染，有乙酰胆碱受体抗体阳性重症肌无力病史，接受霉酚酸酯治疗22年。他出现左眼视力变化和认知记忆缺陷。脑部MRI显示左延髓内有一强化病灶延伸至小脑。脑脊液分析显示爱泼斯坦-巴尔病毒（EBV）阳性，恶性肿瘤阴性。他被诊断为水痘带状疱疹病毒血管病。3个月后，重复脑部MRI显示双侧脑室周围白质出现多个新的强化病灶。不久后，他因急性左侧视力模糊和记忆力减退加重就诊于当地急诊室，并开始接受类固醇治疗。由于症状迅速进展，他接受了左额叶病变切除术，术后病理显示为EBV诱导的弥漫性大B细胞淋巴瘤（DLBCL）。停用霉酚酸酯，在静脉注射一剂500mg/m利妥昔单抗后24小时内，他的左眼视力和记忆力减退有了显著改善。3个周期后，他对利妥昔单抗的反应不一。在接受一剂大剂量甲氨蝶呤后，他随后出现慢性肾病并需要透析。他接受了全脑放疗和颅脊髓放疗，目前处于完全缓解状态。对于脑室周围病变且脑脊液EBV阳性的患者，应高度考虑EBV诱导的DLBCL诊断。由于疾病表现和影像学特征不典型而导致PCNSL诊断的误诊或延迟，可能会导致PCNSL进展和神经功能缺损恶化。
